A gene signature for a long-term survivor of an atypical teratoid/rhabdoid tumor
Copyright © 2014 Elsevier Inc. All rights reserved..
Atypical teratoid/rhabdoid tumors (AT/RTs) are aggressive brain tumors that are commonly associated with a dismal prognosis. However, there have been isolated reports of long-term survival that was not necessarily correlated with other prognostic factors such as age, clinical stage, or extent of surgical resection. Here, we report the case of a 6-year-old boy with AT/RT who remained disease-free for 8 years after undergoing subtotal surgical resection followed only by radiation therapy. On recurrence, the tumor rapidly progressed, leading to the patient's death a short time later. To further characterize this case and learn more about the tumor biology of long-term survivors, we compared the gene expression (GE) profiles from representative samples obtained from primary, recurrent, and progressive disease tumors of the above-mentioned patient along with a cohort of primary untreated AT/RT samples using cDNA microarrays. Global GE analysis and unsupervised hierarchical clustering showed the three events clustered together and distinctly apart from the rest of the samples. This indicates a GE background that is maintained throughout the course of the disease. This unique case suggests that there may be specific clinical characteristics associated with distinctive molecular subtypes of AT/RT. The identification and characterization of AT/RT subtypes could lead to advances in both prognosis and treatment of these tumors.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2014 |
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| Erschienen: |
2014 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:207 |
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| Enthalten in: |
Cancer genetics - 207(2014), 9 vom: 22. Sept., Seite 420-4 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Sredni, Simone T [VerfasserIn] |
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| Links: |
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| Themen: |
AT/RT |
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| Anmerkungen: |
Date Completed 01.04.2015 Date Revised 25.11.2016 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.cancergen.2014.05.009 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM240329635 |
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| 245 | 1 | 2 | |a A gene signature for a long-term survivor of an atypical teratoid/rhabdoid tumor |
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| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2014 Elsevier Inc. All rights reserved. | ||
| 520 | |a Atypical teratoid/rhabdoid tumors (AT/RTs) are aggressive brain tumors that are commonly associated with a dismal prognosis. However, there have been isolated reports of long-term survival that was not necessarily correlated with other prognostic factors such as age, clinical stage, or extent of surgical resection. Here, we report the case of a 6-year-old boy with AT/RT who remained disease-free for 8 years after undergoing subtotal surgical resection followed only by radiation therapy. On recurrence, the tumor rapidly progressed, leading to the patient's death a short time later. To further characterize this case and learn more about the tumor biology of long-term survivors, we compared the gene expression (GE) profiles from representative samples obtained from primary, recurrent, and progressive disease tumors of the above-mentioned patient along with a cohort of primary untreated AT/RT samples using cDNA microarrays. Global GE analysis and unsupervised hierarchical clustering showed the three events clustered together and distinctly apart from the rest of the samples. This indicates a GE background that is maintained throughout the course of the disease. This unique case suggests that there may be specific clinical characteristics associated with distinctive molecular subtypes of AT/RT. The identification and characterization of AT/RT subtypes could lead to advances in both prognosis and treatment of these tumors | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a AT/RT | |
| 650 | 4 | |a Rhabdoid | |
| 650 | 4 | |a gene expression | |
| 650 | 4 | |a long-term survivor | |
| 700 | 1 | |a Huang, Chiang-Ching |e verfasserin |4 aut | |
| 700 | 1 | |a Pundy, Tatiana |e verfasserin |4 aut | |
| 700 | 1 | |a Patel, Kashyap |e verfasserin |4 aut | |
| 700 | 1 | |a Halpern, Abby L |e verfasserin |4 aut | |
| 700 | 1 | |a Grupenmacher, Alex T |e verfasserin |4 aut | |
| 700 | 1 | |a Chou, Pauline M |e verfasserin |4 aut | |
| 700 | 1 | |a Bonaldo, Maria de Fátima |e verfasserin |4 aut | |
| 700 | 1 | |a Tomita, Tadanori |e verfasserin |4 aut | |
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